What is a Moyamoya Disease?

Moyamoya disease is a rare cerebrovascular disorder characterized by the narrowing or blocked arteries at the base of the brain, specifically in the area known as the basal ganglia. This narrowing results in reduced blood flow to the brain, which can lead to a variety of neurological symptoms and potentially cause stroke. “Moyamoya” means “puff of smoke” in Japanese, describing the appearance of the abnormal blood vessels that form as a compensatory response to the reduced blood flow.

What Causes Moyamoya Disease?

  • The exact cause of Moyamoya disease is not well understood.
  • Genetic Factors: There is a genetic predisposition, as the condition sometimes runs in families.
  • Secondary Moyamoya: In some cases, Moyamoya disease can develop as a result of an underlying condition such as sickle cell anemia, neurofibromatosis, or radiation therapy.

What are the Symptoms of Moyamoya Disease?

  • Ischemic Symptoms: Transient ischemic attacks (mini-strokes) or actual strokes can lead to symptoms such as weakness, numbness, or paralysis on one side of the body.
  • Seizures: Moyamoya disease can trigger seizures.
  • Headaches: Severe headaches, often described as migraines, can occur.
  • Cognitive Changes: Changes in cognitive function, memory, and attention.
  • Hemorrhage: In advanced cases, rupture of the abnormal blood vessels can lead to bleeding in the brain.

    What are the Treatment Options for Moyamoya Disease?

    • Surgical Bypass: The primary treatment for Moyamoya disease involves surgical bypass procedures to restore blood flow to the brain.
    • Indirect Bypass: Blood vessels from the scalp are connected to the brain’s surface, allowing blood to bypass the narrowed arteries.
    • Direct Bypass: A blood vessel is directly connected to an artery in the brain.
    • Medical Management: Medications may be prescribed to manage symptoms, control blood pressure, and reduce the risk of complications.

      If you or someone you know is suspected of having Moyamoya disease, it’s important to consult a healthcare provider, preferably a neurologist or neurosurgeon, for proper evaluation, diagnosis, and treatment recommendations. Early diagnosis and appropriate management can help prevent complications and improve outcomes.